摘要
连续静脉注射依前列醇提高运动能力,血流动力学和生存在严重原发性肺动脉高压。肺动脉高压,也可以在患者的结缔组织疾病危及生命。在未来的开放monocentre不受控制的研究中,依前列醇的影响,严重的患者肺动脉高压继发于结缔组织病谁是反应迟钝口服血管扩张剂(包括钙通道阻滞剂),并继续在纽约心脏协会进行了评估(NYHA)官能III或IV级,尽管常规的药物治疗。17例患者接受依前列醇与常规治疗(口服抗凝血剂,利尿剂,补充氧气)相关联的便携式输液泵给药。在最初6周的治疗,2(12%)患者死于肺水肿(N = 1)和严重脓毒症(N = 1)的。在剩下的十五个科目,临床和血流动力学参数6周显著改善。这些患者依前列醇的起始之后随后监测80 +/- 48(范围14-154)周。五(33%)患者死于右心脏衰竭(N = 2),严重的败血症(N = 2)或晕厥(N = 1)和两个病人依前列醇开始后24周和52周进行了成功移植的,。其余8名病人七有一个持续的临床改善。 Short-term epoprostenol therapy is effective in some patients with connective tissue diseases as demonstrated by better clinical status and haemodynamics at six weeks. However, this study reports several cases of early and late major complications including severe sepsis and pulmonary oedema. Additional information is needed to evaluate the benefit: risk ratio of long-term epoprostenol therapy in pulmonary hypertension secondary to connective tissue diseases.